Enfermedad de Addison en un niño con adrenoleucodistrofia ligada al cromosoma X / Primary adrenal failure associated to X-linked adrenoleukodystrophy: report of one case

Primary adrenal failure (PAF) can be congenital or acquired. X-linked adrenoleukodystrophy (ALD-X), produced by the mutation of the ABDC1 gene (Xq28), that leads to the plasma accumulation of very long chain fatty acids, is one of the congenital diseases associated to adrenal destruction. We report a 7 years old boy with fast progression of right strabismus and general symptoms as weariness, weakness and mucosal and skin pigmentation. A brain magnetic resonance image showed a leukoencephalopathy, characteristic of ALD-X. Low plasma cortisol, high ACTH levels and lack of response to ACTH test, confirmed the diagnosis of primary adrenal insufficiency. High plasma levels of C26:0 fatty acids, and C24/22, C26/22 ratios confirmed ALD-X.

Embarazo heterotópico: reporte de un caso / Heterotopico pregnancy: report of a case

El embarazo heterotópico ocurre cuando la implantación de dos o más óvulos fecundados se da simultáneamente en sitios distintos intra y extrauterino o extrauterinos. La tasa de mortalidad fetal es de un 65 por ciento a 95 por ciento y la mortalidad materna es menor al 1 por ciento. Como factores de riesgo reconocidos están las técnicas de reproducción asistida, la enfermedad pélvica inflamatoria, antecedentes de embarazo ectópico previo y cirugía tubárica previa. Se describe aquí el caso de un embarazo heterotópico espontáneo en una paciente de 22 años que presentó a las 10 semanas más 3 días de amenorrea, cuadro abdominal agudo, encontrándose gestación intrauterina y masa anexial izquierda. La vista ultrasonográfica reveló la presencia de embarazo intra y extrauterino, la video laparoscopía y el reporte patológico de los restos placentarios y el legrado, confirmaron el diagnóstico previo. Tres aspectos importantes de esta patología son: su baja incidencia, el ultrasonido como una importante arma diagnóstica y que el tratamiento es quirúrgico.

The heterotopic pregnancy occurs when the implantation of two or more fertilizel ovum are implanted simultaneously intra and extra uterine or both extra uterine. The fetal mortality rate is between 65% to 95% and the maternal mortality rate is less than 1%. The main risk factors are asisted reproduction techniques, pelvic infl ammatory disease, previous ectopic pregnancy and previous tubal surgery. In this study we describe one case of heterotopic pregnancy without risk factors, in a twenty two years old. She was 10. 3 weeks of gestation, with an acute pelvic pain. The ultrasound showed one incomplete intra uterine pregnancy and a left tubal pregnancy. The video laparoscopy and the pathology report confirmed the diagnosis. Three important aspects from this pathology are: the low incidence, the ultrasound as an important tool and the defi nite surgical treatment.

Effects of betamethasone, sulindac and quinacrine drugs on the inflammatory neoangiogenesis response induced by polyurethane sponge implanted in mouse

In this study, we showed the effect of the betamethasone, sulindac and quinacrine alone or combined, on the inflammatory angiogenesis promoted by polyurethane sponge on mice. The main finding reported here is that the formation of new blood vessels was strongly inhibited by low concentration of betamethasone, sulindac or quinacrine, whether alone or in combination. It is known that steroidal anti-inflammatory drugs inhibit the enzymes required for the production of prostaglandins through a nuclear glucocorticoid receptor (GR) mediated mechanism. This mechanism may occur in endothelial cells as well. Considering that activity of cyclo-oxigenases 1 and 2 is inhibited by sulindac, and that these enzymes are located in the stromal tissue, we propose that the anti-angiogenic effect of these agents may occur via inhibition of both COX isoforms. On the other hand, quinacrine inhibited PLA2 activity, and we propose here that the anti-angiogenic effect occurs via inhibition of the enzyme PLA2. The potentiated effect of the association of betamethasone, sulindac and quinacrine may have some therapeutic benefit in the control of pathological angiogenesis. Further studies are required to validate these propositions

Effect of cholinergic agonists on muscular tonus of the lizard small intestine and esophagus

The underlying mechanisms of acetycholine-induced intestinal relaxation in the lizard Liolaemus tenuis tenuis are still unknows. By using a classical model of intestinal recording of isometric contraction and relaxation in conjunction with specific pharmacological tools, this article studies the possible influence of EDRF/NO and nicotinic ganglionar receptors on the Ach-induced relaxation in an effort to elucidate the probable mechanisms involved in ACh effect. It was observed that the relaxation of the lizard intestine elicited by ACh (10(-7) - 4 x 10(-4) M) was not affected by hexametonium (5 x 10(4) M) or tetrodotoxin (10(-6) M). Nicotine (10(-7) to 10(-4) M) induced relaxation was significantly antagonized by hexametonium; however, it was not influenced by tetrodotoxin. These results allow us to discard a neuronal pathway in cholinergic-induced relaxation, suggesting a more direct cholinergic effect on the smooth muscle, perhaps mediated by an unknown substance released by some specialized tissue. N-nitro-L-arginine, used to block NO-synthase and NO production, induced no changes in ACh-induced relaxation. Methylene blue, a soluble guanylate cyclase inhibitor, induced no changes in ACh-induced relaxation. These results allow us to dicard a probable role of EDRF/nitric oxide in the ACh-induced relaxation of lizard small intestine, providing evidence that this mechanism could be different from reported on other species.

Traumatismo abdominal: técnicas diagnósticas ultrasonográficas / Abdominal trauma: ultrasonographic diagnosis

Hay factores que son fundamentales en el manejo del niño traumatizado y en su pronóstico. Algunos de llos son: el estado general y hemodinámico del niño, su edad, el mecanismo y la severidad del traumatismo, la infraestructura médica disponible, los métodos de diagnóstico por imágenes y la experiencia del equipo médico. La determinación exacta de la extensión y gravedad del traumatismo es esencial para el tratamiento y pronóstico del niño y es en este sentido donde los métodos de diagnóstico por imágenes pueden jugar un papel muy importante. Especialmente, si se considera la tendencia actual al tratamiento médico de las lesiones de víscera sólida, donde el impacto clínico de los métodos de imágenes es mayor y la información que éstos aportan influye en la duración de la hospitalización, en la indicación del reposo, en el tiempo y necesidad de seguimiento clínico del paciente